Transcript Case Presentation Conference Children`s Hospital of New Orleans

Case Presentation Conference
Children’s Hospital of New Orleans
James M. Roth M.D.
Evelyn Kluka M.D.
History
• 13 year-old Hispanic male R.G.
• Chief Complaint: Headache, Left Ear Pain
with radiation of the pain to the cheek
History of Present Illness
• 1 month history of progressive left sided
facial pain and tingling
• Recent stuffy nose with clear discharge
• Odynophagia
Past Medical History
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Esophageal Varices
Hematochezia
Jaundice
Cirrhotic liver disease
Past Surgical History
• Liver Transplant 6 months prior to
admission
• Left myringotomy by an ENT in Dallas
secondary to disequilibrium, tinnitus, and
serous fluid collection
Medications
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Bactrim- prophylaxis
Ganciclovir- prophylaxis
Procardia XL
Magnesium
Prednisone
Neoral- Cyclosporine anti-rejection drug
Cellcept-
Allergies/ Immunizations
• No known drug allergies
• No immunizations since liver transplant
• Immunizations up to date till then
Social History
• Born in Mexico
• Lives with mother currently in Dallas
Physical Exam
• Vital Signs normal
• General: Awake alert
• Ears: Right TM clear; Left TM slightly
reddened with some fluid present
• Nose: Reddened inferior turbinates no
drainage
Physical Exam
• Oropharynx: Tonsils 1-2+ symmetric, uvula
midline normal tongue mobility tongue soft
to palpation
• Neck: Small < 1 cm nodes scattered
throughout neck
• Face: Slight swelling to the left midface
Physical Exam
• Neurological: V2 and V3 with decreased
sensation on the left side. Remaining
cranial nerves grossly intact.
Admission
• Originally evaluated Dallas and CT scan
showed a nasal mass
• Admitted by GI/Transplant team and ENT
service was consulted for biopsy
MRI
• Mass filling the nasopharynx compressing
or encompassing the left Eustachian tube
with area of central necrosis
Intraoperative Findings
• Fungating gray mass filling most of the
nasopharynx slight more on the left than the
right
• Very solid in nature and avascular
Lab Work
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EBV titers IgM elevated
CBC wnl
Chem 7 wnl
PT/PTT wnl
Surgical Pathology
• Large lesion 3.5x1.5x.5 cm
• Lymphoid lesion
• Polyclonal cells: small mature lymphocytes,
large active immunoblast, T cells, B cells,
Strongly EBV positive
Diagnosis
• Post Transplant Lymphoproliferative
Disease (PTLD): Polyclonal Variant
PTLD
• The presence of an abnormal proliferation of
lymphoid cells
• Highly related to EBV infection
• Related to the type of solid organ
transplanted
• More common in children
• Originally described in 1969 in 5 renal
transplant patients
Pathology
• Several variants from benign polyclonal B
cell hyperplasia to malignant monoclonal
lymphoma
• The progression to a monoclonal population
leads to a more aggressive and malignant
tumor
Why transplant patient’s?
• Immunosuppression is targeted against T
cells especially cytotoxic T cells
• These cells help to self regulate the immune
system
• With certain viral infection you get B cell
proliferation
• These cells can progress in an unregulated
manner
EBV Infection
• Causes an active B cell proliferation
• Linked to Burkitt’s lymphoma and
nasopharyngeal cancer
• R.G. was originally seronegative prior to
transplantation
• His runny nose and sore throat may have
represented a recent EBV infection
Common Presentation
• Mononucleosis type infection
• Febrile illness with leukopenia
• Focal organ system failure
– GI tract: endoscopy, CT scans
– CNS: lumbar puncture
– Lymph node involvement
Solid Organ Transplant
• Renal- 1%
• Liver- 2-3%
• Heart- 4-10%
Risk Factors
• Young age: Increased risk of primary EBV
infections in the early post transplant period
• Agents: Not any single agent more
responsible but the cumulative intensity of
immunosuppression seems to be most
important.
Treatment
• Decrease immunosuppression
• Antivirals: acyclovir ganciclovir
• Immunoglobulins: IVIG which helps to target
CMV
• Chemotherapuetics: Rituximab (CD20 ligand)
• Radiotherapy
Conclusions
• PTLD is a rare complication of
transplantation- 2% of all solid organ
transplant recipients
• More common in children secondary to
primary exposure to EBV
• May present in the head and neck especially
do to the rich lymphatic system
• Treatable as long as there is not monoclonal
proliferation
R.G.
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Underwent treatment at a variety of levels
Immunosuppression was decreased
Given IVIG, Acyclovir, Ganciclovir
Started on Rituximab
Received radiation therapy treatments
Repeat MRI did eventually show regression
of disease